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Signs, complications, and platelet aggregation in familial exudative vitreoretinopathy
1Department of Ophthalmology, Canisius Wilhelmina Hospital, Nijmegen, The Netherlands.
American Journal of Ophthalmology
|January 15, 1991
Summary
Familial exudative vitreoretinopathy (FEVR) is a genetic disorder affecting retinal vascular development. Complications like retinal detachment significantly impair vision, with limited surgical success.
Area of Science:
- Ophthalmology
- Medical Genetics
- Retinal Diseases
Background:
- Familial exudative vitreoretinopathy (FEVR) is a rare inherited condition.
- It is characterized by abnormal retinal vascular development, leading to vision-threatening complications.
Purpose of the Study:
- To investigate the clinical manifestations and outcomes of familial exudative vitreoretinopathy (FEVR).
- To explore the pathogenesis of FEVR.
Main Methods:
- Retrospective analysis of 106 patients (16 pedigrees) with FEVR diagnosed between 1979 and 1989.
- Clinical examination, assessment of visual acuity, and documentation of complications.
- Platelet aggregation studies in a subset of patients and controls.
Main Results:
- 101 patients had familial FEVR, 5 had sporadic FEVR.
- Common complications included posterior retinal deformation, vitreous hemorrhage, amblyopia, and retinal detachment.
- Retinal detachment occurred in 21% of eyes, with successful surgical reattachment in only 50% of operated eyes.
- Retinal neovascularization (11%) and exudates (9%) were also observed.
Conclusions:
- FEVR is associated with significant visual impairment due to complications.
- The primary pathogenesis involves a premature arrest in retinal vascular development.
- Current surgical interventions for FEVR-related retinal detachment have limited success rates.